CASE REPORTS   

Giornale Italiano di Dermatologia e Venereologia 2007 October;142(5):603-5

Copyright © 2007 EDIZIONI MINERVA MEDICA

language: English

Treatment of familial benign pemphigus with topical tacrolimus: an effective therapy?

Gallo L., Lo Presti M., Ayala F.

Unit of Dermatology, Allergology and Venereology Department of Pathology University “Federico II” of Naples, Naples, Italy

Familial benign pemphigus (FBP) is a rare autosomal-dominant genodermatosis, with incomplete penetrance. FBP frequently appears in the late teenage years or in adulthood (30s and 40s). It is characterized by recurrent skin eruptions, mostly in the intertriginous areas. The clinical features are represented by vesicles that progress to fissured erythematous plaques, bullae and erosions with overlying crusts in the axillary, neck, genital and chest areas. FBP can be a debilitating condition, both physically and psychologically. Patients suffer from pruritus, burning, intense pain and restricted mobility. Despite a wide variety of topical and systemic medical treatments, the disease presents a major therapeutic challenge due to a high recurrence rate. A variety of topical and systemic treatments have been used, and in particular corticosteroids, which are often limited in their use by secondary effects and localization of the lesions. We describe the case of a 38 -year old woman with a five-year history of FBP whom was unresponsive to systemic and local corticosteroids and treated with topical tacrolimus 0.03% twice a day with early improvement of the disease. Thereafter, she was also treated with eosin, oral antibiotics and topical corticosteroids because of lack of complete control induced by tacrolimus. Nevertheless, we believe that tacrolimus could be employed as an efficacious therapeutic tool for FBP, even in the acute phase of disease. More cases have to be treated in order to confirm its efficacy in patients resistant to conventional therapy.